Adachi H, Tsuboi M, Hayashi K
Department of Neurosurgery, Bizen Municipal Hospital, 2245 Ibe, Bizen-city, Okayama 705-0001, Japan.
No Shinkei Geka. 2001 Jul;29(7):659-65.
We report a case of a 64-year-old man with intravascular lymphomatosis. He had dementia and generalized seizure. Computed tomogram scan and magnetic resonance imaging showed multiple infarctions in the right frontal lobe, right cerebellar hemisphere and left occipital lobe. A month after admission he had a daily high fever and frequent convulsions. The level of serum LDH was progressively getting higher and the level of beta 2-microglobulin in both serum and CSF was high. One and a half months after admission we performed an open biopsy for making a definite diagnosis by pathological examination. Histological diagnosis was intravascular lymphomatosis (angiotrophic lymphoma) of large B cell type. He was treated with regimens of combined chemotherapy with M-CHOP (methotrexate, cyclophosphamide, adriamycin, vincristine, prednisolone). After chemotherapy his consciousness was gradually improving better and the level of serum LDH and beta 2-microglobulin was reduced to a low level. Three months after admission he died of respiratory failure. After a review of the literature and experience with this case, it is obrious that early diagnosis of this disease is difficult. However, we think that multiple cerebral infarctions on neuroradiological examinations and high levels of serum LDH (especially LDH2 and LDH3) and beta 2-microglobulin in CSF and serum should lead to a presumptive diagnosis of this disease, and biopsy should be used to make a difinite diagnosis.
我们报告一例64岁患有血管内淋巴瘤的男性患者。他患有痴呆和全身性癫痫。计算机断层扫描和磁共振成像显示右额叶、右小脑半球和左枕叶有多处梗死。入院一个月后，他每天高热且频繁抽搐。血清乳酸脱氢酶水平逐渐升高，血清和脑脊液中的β2-微球蛋白水平都很高。入院一个半月后，我们进行了开放性活检，以便通过病理检查做出明确诊断。组织学诊断为大B细胞型血管内淋巴瘤（嗜血管性淋巴瘤）。他接受了M-CHOP（甲氨蝶呤、环磷酰胺、阿霉素、长春新碱、泼尼松龙）联合化疗方案的治疗。化疗后，他的意识逐渐好转，血清乳酸脱氢酶和β2-微球蛋白水平降至低水平。入院三个月后，他死于呼吸衰竭。在回顾了该病例的文献和经验后，很明显这种疾病早期诊断困难。然而，我们认为神经放射学检查发现的多处脑梗死以及脑脊液和血清中高水平的血清乳酸脱氢酶（尤其是乳酸脱氢酶2和乳酸脱氢酶3）和β2-微球蛋白应导致对该疾病的推测性诊断，并且应通过活检做出明确诊断。
